Abstract Congenital urethral fistula is an extremely rare but easily manageable anomaly that may be confused with hypospadias. This is a case description of a ...
Pediatr Surg Int (2000) 16: 386±387
Ó Springer-Verlag 2000
ORIGINAL ARTICLE
Man Mohan Harjai
Congenital urethrocutaneous ®stula
Accepted: 26 May 1999
Abstract Congenital urethral ®stula is an extremely rare but easily manageable anomaly that may be confused with hypospadias. This is a case description of a congenital ®stula of the anterior urethra. Awareness of the entity will avoid complications. Key words Urethrocutaneous ®stula á Anorectal malformations á Hypospadias
Introduction Male congenital urethral ®stula (CUF) is most commonly seen in association with anorectal malformations (ARM) [1]. However, congenital posterior urethroperineal ®stula as a variant of urethral duplication is a relatively more common type of ®stula. Isolated CUF is very rare, with less than 20 cases reported in the available literature. We report such a case and discuss its management.
Case report A 3-year-old boy was admitted with passage of urine from the undersurface of the penis as well as the tip since birth. He had no history of an injury or operations in the past. On examination, he had a patent distal urethra and a normal prepuce. There was no chordee. The urethra distal to the ®stula was surrounded by corpus spongiosum. An opening was present on the ventral aspect of the midshaft of the penis. A catheter could be passed through the ®stula and out of the normally-situated meatus at the glans. Both testicles were in the scrotum; there were no other associated anomalies (Fig. 1). Abdominal ultrasonography and a micturating cystourethrogram were normal. The ®stula was
M. M. Harjai Department of Surgery, Armed Forces Medical College, Pune-411040, India
closed with a preputial onlay ¯ap. There was abundant tissue present to form an additional layer before skin closure. A small silicon catheter was left in the bladder for drainage, and was removed on the 5th postoperative day. The patient is doing well without recurrence.
Discussion Isolated congenital ®stula of the penile urethra without hypospadias or chordee is very rare, and thus, even senior residents may misdiagnose it as hypospadias without chordee. The urethra develops from the caudal end of the urogenital sinus. Olbourne suggested that the formation of a CUF is due to a focal or temporary defect in the urethral plate, which prevents fusion of the urethral folds [2]. Cook and Stephens suggested an alternative mechanism that should be considered, namely, pressure atrophy from the heel of the baby's foot, leading to pressure necrosis [3]. The distal glanular penile urethra is normal as it develops from a surface ectodermal invagination [4]. Karnak et al. regarded congenital urethrocutaneous ®stulae (excluding those associated with ARM and true duplications) as one set of anomalies [5]. Before planning the surgical approach, it is important to exclude urethral duplication, which produces a Y-type ®stula with dierent management implications. Treatment of CUF depends on the associated penile abnormality and ®stula location. If the urethra beyond the ®stula is congenitally defective, then simple closure is usually unsuccessful and the ®stula is likely to recur [6]. If adequate lateral skin is present, repair can be carried out as an one would perform a second-stage Johanson urethroplasty. Otherwise, an onlay ¯ap can be used. In summary, CUF is an extremely rare but easily manageable anomaly, which can readily be confused with hypospadias. Awareness of the entity will help to avoid complications.
387 2. Olbourne NA (1976) Congenital urethral ®stula. Plast Reconstr Surg 57: 237±238 3. Cook WA, Stephens FS (1990) Pathoembryology of the urinary tract. In: King LR (ed) Urological surgery in neonates and young infants. Saunders, Philadelphia, pp 724±725 4. Ritchy ML, Sinha A, Argueso L (1994) Congenital ®stula of the penile urethra. J Urol 151: 1061±1062 5. Karnak I, Tanyel FC, Hicsonmez A (1995) Congenital urethrocutaneous ®stula: a case report and literature review, with a nomenclature proposal. J Pediatr Surg 30: 1504±1505 6. Maara®e A, Azmy AF (1997) Congenital ®stula of the penile urethra. Br J Urol 79: 814
Fig. 1 Congenital urethral ®stula on ventral mid-penile shaft with catheter exiting through ®stula and normal meatus at glans
References 1. Stephens FD (1983) Congenital malformations of the urinary tract. Praeger, New York, pp 128±130
